Familial Mediterranean Fever
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What is familial Mediterranean fever?
Familial Mediterranean fever is an inherited condition characterized by recurrent episodes of painful inflammation in the abdomen, chest, or joints. These episodes are often accompanied by fever and sometimes a rash. The first episode usually occurs by the age of 20, but in some cases, the initial attack occurs much later in life. Typically, episodes last 12 to 72 hours and can vary in severity and length of time between attacks. A buildup of protein deposits (amyloidosis) occurs in some cases of familial Mediterranean fever and can lead to kidney failure if left untreated.
How common is familial Mediterranean fever?
Familial Mediterranean fever primarily affects populations originating in the Mediterranean region, particularly people of Armenian, Arabic, Turkish, and Jewish ancestry. The frequency of affected individuals in these populations ranges from 1 in 250 people to 1 in 1,000 people. This disorder is less common in other populations.
What genes are related to familial Mediterranean fever?
Mutations in the MEFV gene cause familial Mediterranean fever.
Normal variations in the SAA1 gene modify the course of familial Mediterranean fever.
The MEFV gene provides instructions for making a protein called pyrin (also known as marenostrin), which is found in white blood cells. This protein probably helps to limit or stop the inflammation process. Mutations in the MEFV gene can lead to reduced activity of the pyrin protein, which disrupts control of the inflammation process. With impaired control, an inappropriate or prolonged inflammatory response can occur, usually accompanied by fever and pain in the abdomen, chest, or joints.
Some evidence suggests that a particular version of the SAA1 gene (called the alpha variant) may increase the risk of amyloidosis among people with familial Mediterranean fever.
How do people inherit familial Mediterranean fever?
This condition is inherited in an autosomal recessive pattern, which means two copies of the MEFV gene in each cell must be altered for a person to be affected by the disorder. Most often, the parents of an individual with an autosomal recessive disorder are carriers of one copy of the altered gene but do not show signs and symptoms of the disorder.
In rare cases, familial Mediterranean fever appears to be inherited in an autosomal dominant pattern, which means one copy of an altered MEFV gene is sufficient to cause the disorder. In these cases, an affected person has one parent with the condition.
Source: National Institutes of Health
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Studies in the area of familial mediterranean fever therapy reported from Ondokuz Mayis University, Department of Periodontology
2009 JUN 1 - (NewsRx.com) -- New investigation results, 'Periodontal disease in patients with familial Mediterranean fever: from inflammation to amyloidosis,' are detailed in a study published in Journal of Periodontal Research. "Familial Mediterranean fever stimulates a very intense acute-phase reactants response and if left untreated eventually leads to amyloidosis. The aim of this study was to determine the prevalence of periodontal disease among patients with familial Mediterranean fever in the Black Sea region in Turkey and to evaluate whether periodontitis is related to amyloidosis in patients with familial Mediterranean fever," scientists in Samsun, Turkey report. "One-hundred and thirty three patients with familial Mediterranean fever and 50 healthy subjects were included in this study. Periodontal health and disease were evaluated using the gingival index, papillary bleeding index, plaque index and periodontal disease index. The concentrations of serum acute-phase reactants were measured at baseline and at 4-6 wk after completion of the nonsurgical periodontal therapy. Genetic testing for familial Mediterranean fever was performed using the familial Mediterranean fever StripAssay. Kidney biopsy was carried out on all proteinuric patients. The prevalence of moderate to severe periodontitis in familial Mediterranean fever patients with amyloidosis (80.6%) was significantly greater (p <0.01) than in familial Mediterranean fever patients without amyloidosis (38%) and in controls (20%). Serum levels of acute-phase reactants in familial Mediterranean fever patients were reduced significantly following nonsurgical periodontal therapy (p <0.01). Periodontal therapy seems to reduce the serum levels of acute-phase reactants in patients with familial Mediterranean fever," wrote M.I. Cengiz and colleagues, Ondokuz Mayis University, Department of Periodontology. The researchers concluded: "Therefore, treating periodontitis might help to alleviate the disease burden in patients with familial Mediterranean fever." Cengiz and colleagues published their study in the Journal of Periodontal Research (Periodontal disease in patients with familial Mediterranean fever: from inflammation to amyloidosis. Journal of Periodontal Research, 2009;44(3):354-61). For more information, contact M.I. Cengiz, Ondokuz Mayis University, Dept. of Periodontology, Samsun, Turkey. Publisher contact information for the Journal of Periodontal Research is: Blackwell Publishing Inc., 350 Main St., Malden, MA 02148, USA. Keywords: Turkey, Samsun, Familial Mediterranean Fever Therapy, Amyloidosis, Familial Mediterranean Fever, Inflammation, Nephrology, Periodontal Disease, Periodontal Research, Periodontitis, Periodontology, Rheumatology, Therapy, Treatment. This article was prepared by Biotech Business Week editors from staff and other reports. Copyright 2009, Biotech Business Week via NewsRx.com.
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